Department of Neurosurgery, Hospital de Especialidades, Centro Médico Nacional Siglo XXI, 06720 Mexico City, DF, Mexico ; Centro Neurológico ABC, 05300 Mexico City, DF, Mexico.
Int J Endocrinol. 2012;2012:306094. doi: 10.1155/2012/306094. Epub 2012 Nov 12.
Acromegaly is a complex disease that requires the intervention of a multidisciplinary team. The most frequent clinical manifestations are growing of distal parts of the body and some areas of the face. Patients may also present arterial hypertension, diabetes mellitus, colonic polyps, cardiomegaly, neurological and endocrine changes secondary to the presence of a GH-secreting tumor in pituitary or extrapituitary origin, or eutopic hypothalamic GHRH hypersecretion and peripheral GHRH hypersecretion. Surgery is the first treatment used for most patients, regardless of the cause. In the great majority of cases, pituitary tumor can be removed through a transsphenoidal approach. Craniotomy is reserved for those cases with giant tumors, particularly when they grow toward the middle or posterior cranial fossa. Best surgical results are obtained when the tumor is confined into the sella turcica or if it has a regular suprasellar extension. When the disease cannot be controlled with surgery, medical treatment is indicated. Somatostatin analogues are included as the first line of medication, followed by dopamine agonist and growth hormone receptors antagonists. Radiation therapy can be also indicated in two main forms for residual tumor with medically refractory patients: radiosurgery for small tumors or fractionated stereotactic radiotherapy for larger ones.
肢端肥大症是一种复杂的疾病,需要多学科团队的干预。最常见的临床表现是身体远端和面部某些部位的生长。患者还可能出现动脉高血压、糖尿病、结肠息肉、心脏扩大、继发于垂体或垂体外起源的生长激素分泌瘤的神经和内分泌变化,或下丘脑 GHRH 异位分泌和外周 GHRH 分泌过多。手术是大多数患者的首选治疗方法,无论病因如何。在绝大多数情况下,通过经蝶窦入路可以切除垂体瘤。开颅手术仅适用于巨大肿瘤的病例,特别是当肿瘤向颅中窝或颅后窝生长时。当肿瘤局限于蝶鞍内或具有规则的鞍上延伸时,手术效果最佳。当疾病不能通过手术控制时,需要进行药物治疗。生长抑素类似物被列为一线药物,其次是多巴胺激动剂和生长激素受体拮抗剂。对于药物难治性患者的残余肿瘤,还可以采用两种主要形式的放射治疗:小肿瘤的放射外科治疗或大肿瘤的分次立体定向放射治疗。